OBJECTIVE: Total anomalous pulmonary venous repair in univentricular physiology is associated with a high mortality risk and often these patients are not considered surgical candidates. Therefore we sought to evaluate outcomes of concomitant repair in single ventricle (SV) patients with total anomalous pulmonary venous return (TAPVR) lesions.METHODS: A single center retrospective chart was performed from Sept 2017 to Jun 2019 to identify patients with the concomitant diagnoses of SV and TAPVR who underwent surgical repair. Data regarding preoperative, intraoperative, and postoperative patient characteristics were collected and analyzed.RESULTS: A total of five patients (four male, one female) with SV and TAPVR were operated upon, at a mean age and weight of 12.8 + 11.3 days and 3.6 + 0.5 kg respectively. Preoperative patient characteristics included prematurity in two, heterotaxy syndrome in four, and obstructed TAPVR in two patients. The subtype of TAPVR was supracardiac in two and intracardiac in three patients. SV anatomy was hypoplastic left heart syndrome (HLHS) in one, tricuspid atresia (TA) and pulmonary atresia (PA) in one, and an unbalanced atrioventricular septal defect (UAVSD) in three patients. There were no early deaths (30 day mortality) and 100% of patients progressed to stage II palliation. At last follow up (mean 327 + 94.8 days), all patients are alive.CONCLUSIONS: Despite historically poor outcomes, patients with concomitant SV and TAPVR lesions are potential operative candidates as evidenced by this case series, however larger patient cohort and longer term follow up are warranted.